HPA019366
Anti-LRBA antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1
别名:
LRBA Antibody - Anti-LRBA antibody produced in rabbit, Lrba Antibody, Anti-Beige-like protein, Anti-CDC4-like protein, Anti-Lipopolysaccharide-responsive and beige-like anchor protein
产品名称
Anti-LRBA antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
immunogen sequence
SVLMVSKYRDILEPQNERHSQSCTETGSENENVSLSEITPAAFSTLTTASVEESESTSSARRRDSGIGEETATGLGSHVEVTPHTAPPGVSAGPDAISEVLSTLSLEVNKSPETKNDRGNDLDTKATPSVSV
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... LRBA(987)
Biochem/physiol Actions
Lrba interacts with protein kinase A and modulates the intracellular vesicle trafficking to activated receptor complexes. This aids the secretion and membrane deposition of immune effector molecules, like cytotoxic T lymphocyte-associated antigen‐4 (CTLA4). The PH-like domain is critical for membrane association and WD-40 domain helps in ligand binding and signaling.
LRBA (lipopolysaccharide-responsive and beige-like anchor protein) participates in endocytosis of ligand-activated receptors. Mutations in LRBA are linked with inflammatory bowel disease (IBD)-like symptoms, autoimmune lymphoproliferative syndrome-like disease and IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome.
Application
Anti-LRBA antibody produced in rabbit has been used:
- in western blotting
- fluorescence-activated cell sorting (FACS)
- basic Local Alignment Search Tool (BLAST) analysis,
- flow cytometry analysis
Anti-LRBA antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
General description
Lrba consists of a BEACH domain situated between a PH-like domain and WD-40 domain.
The gene LRBA (lipopolysaccharide-responsive and beige-like anchor protein) is mapped to human chromosome 4q31.3. It belongs to the BEACH (beige and chediak-higashi)-WD40 (β-transducin) protein family. LRBA is ubiquitously expressed. The protein localizes in the cytoplasm. Upon activation of B cells, LRBA moves to the membrane of trans-Golgi network, lysosomes, endoplasmic reticulum and to the cell surface.
Immunogen
Lipopolysaccharide-responsive and beige-like anchor protein recombinant protein epitope signature tag (PrEST)
Other Notes
Corresponding Antigen APREST74669
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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存储类别
10 - Combustible liquids
wgk
WGK 1
法规信息
常规特殊物品
此项目有
LRBA gene deletion in a patient presenting with autoimmunity without hypogammaglobulinemia.
Siobhan O Burns et al.
The Journal of allergy and clinical immunology, 130(6), 1428-1432 (2012-09-18)
The imbalance of circulating T helper subsets and regulatory T cells in patients with LRBA deficiency: correlation with disease severity
Azizi G, et al.
Journal of Cellular Physiology, 233(11), 8767-8777 (2018)
Rapid flow cytometry-based test for the diagnosis of lipopolysaccharide responsive beige-like anchor (LRBA) deficiency
Gamez-Diaz, Laura et al.
Frontiers in Immunology, 9(3), 720-720 (2018)
Clinical, immunological and genetic characteristic of patients with clinical phenotype associated to LRBA-deficiency in Colombia.
Martinez-Jaramillo C, et al.
columbia medica, 50(3), 176-191 (2019)
Ulrich Salzer et al.
Arthritis research & therapy, 14(5), 223-223 (2012-10-10)
Common variable immunodeficiency (CVID) describes a heterogeneous subset of hypogammaglobulinemias of unknown etiology. Typically, patients present with recurrent bacterial infections of the respiratory and gastrointestinal tract. A significant proportion of CVID patients develops additional autoimmune, inflammatory or lymphoproliferative complications. CVID
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